Postoperative Obstructive Symptoms are Common after Rehbein's Procedure for Hirschsprung's Disease

Abstract

Rehbein's procedure for Hirschsprung's disease has been questioned because several centimetres of aganglionic bowel are left behind, considered to result in obstructive symptoms. However, the reported outcome is similar to that after operations with other techniques. The purpose of this study was to review our experience of Rehbein's procedure during an 8-year period, focusing particularly on obstructive symptoms. We retrospectively studied 44 patients operated with Rehbein's procedure for biopsy-verified Hirschsprung's disease from October 1993 to October 2001. Three patients with total colonic aganglionosis and two patients who underwent total colectomy as a result of severe enterocolitis finally had a distal ileo-rectal anastomosis. Four (9 %) patients had a sphincteromyectomy before the Rehbein procedure. The patients' bowel function was evaluated by a questionnaire and an interview carried out 27 to 123 (mean 82) months after the operation. Two patients could not be traced and one patient, with total colonic aganglionosis, had an ileostomy at follow-up. Nineteen (46 %) of the 41 patients were treated with oral laxatives or enemas (12 patients), required repeated Botox injections (4 patients), or had undergone sphincteromyectomy (9 patients) due to obstructive symptoms. Eleven (27 %) of the 41 evaluated patients had soiling at least once a week at follow-up. Eleven (29 %) of 38 patients with a colorectal anastomosis had good bowel function without additional therapy. In conclusion, Rehbein's procedure for the treatment of Hirschsprung's disease is often complicated by obstructive problems.

Re-operations for Hirschsprung’s Disease: Long-term Complications

“…To avoid a repeat operation because of residual aganglionosis after a Rehbein procedure, the remaining rectum should be short enough and kinking and twisting must be prevented. Because of impaired relaxation of the aganglionic distal rectum, obstructive symptoms are common [ 15 ] . A Rehbein procedure may not be the currently preferred technique for HD.…”

Redo surgery with longitudinal resection for dilated bowel in Hirschsprung disease: an illustrative case series

“…Data on (1) demographic characteristics of patients (i.e. sex, age at primary pull-through, age at longitudinal resection), (2) disease characteristics and clinical course (i.e. length of the initial affected and resected segment, previous interventions), (3) the procedure of longitudinal resection (i.e.…”

“…Hirschsprung disease (HD) is a congenital motility disorder characterized by aganglionosis of the distal colon, for which surgery to resect aganglionic bowel is necessary. Thirty to 50% of patients experience defecation problems after primary surgical treatment [1,2], of which persistent obstructive problems such as constipation are the most common. Possible causes for persistent obstructive complaints include anastomotic stricture, residual aganglionosis, mechanical obstruction due to adhesions, sphincter problems and stool holding behaviour [3].…”

Advances in Hirschsprung Disease Genetics and Treatment Strategies

“…62 Because of long-term development of severe constipation in many patients secondary to retained aganglionic segment it has fallen out of favor. 63…”